原发性肝脏神经内分泌癌的诊断和治疗

Diagnosis and treatment of primary hepatic neuroendocrine carcinoma

目的：探讨原发性肝脏神经内分泌癌的临床特点和诊断治疗方法。方法回顾性分析解放军总医院2009年5月至2013年12月经病理确诊的14例原发性肝脏神经内分泌癌患者的临床资料。结果14例原发性肝脏神经内分泌癌患者中，男8例，女6例；年龄23～58岁，平均年龄45．9岁。肿瘤位于肝右叶4例，肝左叶4例，双叶均有6例。临床症状多为非特异性腹痛或腹胀。影像学检查很难与肝细胞癌区分，诊断需经病理学证实及排除肝外原发灶。所有患者术前的影像学检查、消化内镜以及术后长期随访均未发现肝外原发灶。14例患者中，接受单纯手术切除3例，接受肝肿瘤切除＋射频消融2例，接受经皮肝穿刺肝动脉介入化疗栓塞（ TACE）6例，行同种异体原位肝移植1例，仅行腹腔探查1例，行化疗1例。14例患者均获得随访，至随访结束，生存7例，死于肝衰竭或肿瘤复发7例。结论原发性肝脏神经内分泌癌是一种罕见的肝脏肿瘤，诊断需经病理证实，推荐超声引导下穿刺活检。原发性肝脏神经内分泌癌的预后相对较好，治疗首选手术切除，对于不能切除的患者可选择TACE、射频消融、化疗、肝移植等方法。

Objective To investigate the clinical features, diagnostic and therapeutic methods of primary hepatic neuroendocrine carcinoma. Methods The clinicopathological data of fourteen patients with primary hepatic neuroendocrine carcinoma confirmed by pathology were analyzed retrospectively and related literatures were reviewed. Results The fourteen patients,including eight males and six females, had an age range of 23-58 years （mean 45.9 years）. Four tumors were located in the right liver lobe, four in the left liver lobe and six in both. The clinical manifestations were nonspecific and variable. The most common clinical manifestation was abdominal distention or right upper quadrant pain. Radiological findings were not specific and could not distinguish primary hepatic neuroendocrine tumor from hepatocellular carcinoma. Diagnosis of primary hepatic neuroendocrine tumor was confirmed by pathology using immunohistochemical staining and by the absence of extrahepatic primary lesions. Extrahepatic primary neuroendocrine carcinoma was ruled out by ultrasonography, computed tomography （ CT ） , magnetic resonance imaging （ MRI ） , positron emission tomography-computed tomography （ PET-CT） , preoperative gastrointestinal endoscopy and long-term postoperative follow up. Three patients received surgical treatment, two cases received surgical resection and radiofrequency ablation （RFA）, six patients received transarterial chemoembolization, one case received orthotopic liver transplantation, one case only received exploratory laparotomy, and one case received chemotherapy. All 14 patients were followed up and seven of them are still alive, the others died of liver failure or recurrence. Conclusions Primary hepatic neuroendocrine carcinomas are extremely rare. Its diagnosis should be confirmed by pathology. Preoperative fine needle biopsy is strongly recommended. Prognosis is relatively favorable. Surgical resection is treatment of first choice, and TACE, RFA, and chemotherapy can be used for unresectable patients.